Kurisu Kota, Hida Kazutoshi, Yano Shunsuke, Yamaguchi Shigeru, Motegi Hiroaki, Kubota Kanako, Iwasaki Yoshinobu
Department of Neurosurgery, Hokkaido University School of Medicine, Japan.
No Shinkei Geka. 2008 Dec;36(12):1127-32.
Congenital dermal sinuses, resulting from abnormal neurulation, are relatively uncommon and cause the patients to contract intraspinal infection. Sometimes, this intraspinal infection develops intramedurally abscess with severe neurological deficits. Therefore, a lack of awareness about this problem can result in fatal neurological sequelae. We report the case of a 1-year-old boy who had a lumbo-sacral dimple at birth without further evaluation. He presented repeated fever and rapidly progressive paraplegia. MRI showed a huge intraspinal and intramedullary abscess with dermoid, which was thought to be the result of a dermal sinus. Subsequently, he was transferred to our hospital and received immediate surgery. The patient underwent irrigation of purulent material in the intraspinal abscess including on intramedullary lesion, removal of dermoid tumor, and the resection of the dermal sinus. After that, he was treated with 8 weeks of antibiotic therapy. The patient remained paraplegia but made improvement. This patient illustrates the importance of the recognition and evaluation of skin markers. Prophylactic surgery is indicated to prevent dangerous and recurrent infections of the central nervous system.
先天性皮样窦是由神经胚形成异常引起的，相对少见，可导致患者发生椎管内感染。有时，这种椎管内感染会发展为髓内脓肿并伴有严重的神经功能缺损。因此，对该问题认识不足可能导致致命的神经后遗症。我们报告一例1岁男孩的病例，他出生时腰骶部有一酒窝，未进一步评估。他出现反复发热和快速进展的截瘫。磁共振成像显示巨大的椎管内和髓内脓肿伴皮样囊肿，考虑为皮样窦所致。随后，他被转诊至我院并立即接受手术。患者接受了椎管内脓肿包括髓内病变的脓性物质冲洗、皮样肿瘤切除及皮样窦切除手术。之后，他接受了8周的抗生素治疗。患者仍有截瘫但有所改善。该病例说明了识别和评估皮肤标志物的重要性。预防性手术对于预防中枢神经系统危险的复发性感染具有重要意义。
